Multi-center analysis of calcinosis in children with juvenile dermatomyositis]

OBJECTIVES: To reveal the frequency and the clinical characteristics of dystrophic calcification that occurs in children with juvenile dermatomyositis, multi-center analysis was constructed. METHOD: Fifty children with JDM were enrolled, and 14 of them (28.0%) were complicated with calcinosis. Clinical symptoms and laboratory tests at onset, initial therapy and disease course were compared in children with and without calcinosis. RESULTS: The mean age of the onset of calcinosis was 4.78 +/- 3.33 years, and it was younger than those of children without calcinosis (8.66 +/- 3.85 years) (P = 0.0017). No differences of clinical manifestation except Gower's sign were observed. The frequency of positive anti-nuclear antibody was 7.1% in children with calcinosis and 52.9% without calcinosis (P = 0.0112). The initial therapy of methylprednisolon pulses gave no effects on prognosis of calcium deposition. The calcinosis appeared in 1.56 +/- 1.91 year after the onset of the disease. The various types of calcium deposition including large tumorous clumps, subcutaneous plaques or nodules, sheet-type calcification were deserved. They appeared over knee joints (64.3%), elbow joint (64.3%), and hip processes (50.0%). Calcinosis affecting the subcutaneous tissues frequently resulted in painful superficial ulceration of the overlying skin (42.9%), local infection (50.0%), and limitation of joint movement (14.3%). Although aluminum phosphate was effective in 2 children among 7, no other effective treatment was recommended. In 5 cases, surgical removal of tumorous clumps was operated. Thus, juvenile dermatomyositis is frequently complicated with calcinosis. This type of calcinosis was found to be unlikely to resolve completely, and resulted in severe disability in children.     

ANALYSIS OF BURIED BUMPER SYNDROME AFTER PERCUTANEOUS ENDOSCOPIC GASTROSTOMY DUE TO USE OF A BUTTON‐TYPE KIT

Background: Buried bumper syndrome (BBS) is a rare complication of percutaneous endoscopic gastrostomy (PEG). Along with the widespread use of the button‐type kit, BBS is encountered frequently. Methods: In the present study, we examined causes and treatments for BBS among 1400 patients who had undergone PEG. Results: The causes of BBS after PEG were classified into two categories: early causes consisted of wound infection, inappropriate size of kit and severe lordosis, while late causes were inappropriate exchange of kit, rough management or weight gain. The treatments for BBS could be determined by the degree of wound infection, fistula and burial of the bumper. Conclusion: We prepared a flowchart for replacement, by which BBS can be managed safely and quickly without surgical or endoscopic intervention.     

A case of severe summer-type hypersensitivity pneumonitis treated with high-dose administration of steroid]

A 40-year-old man who lived in a wooden house built 30 years ago presented with complaints of fever, dry cough and dyspnea. Chest X-ray findings showed interstitial shadows throughout bilateral lung fields. After admission, high-dose administration of 3000 mg of methylprednisolone was performed because of deterioration of chest X-ray shadows and symptoms. In a week, clinical data and symptoms improved. Findings of BAL fluid on admission revealed a relative increase of lymphocytes, neutrophils and mast cells, and pathological findings of transbronchial lung biopsy revealed non-caseous granulation and alveolitis. Precipitating antibodies and indirect fluorescent antibodies against Trichosporon cutaneum and Cryptococcus neoformans had positive reactions and T. cutaneum was isolated and identified from the patient's house. A diagnosis of summer-type hypersensitivity pneumonitis was made according to the criteria advocated by Ando et al. This seemed to be a rare case of summer-type hypersensitivity pneumonitis prolonged after isolation from his normal living environment, successfully treated by high-dose administration of steroid.     

Synthesis of 4,5-diarylthiazole derivatives as blood platelet aggregation inhibitors

A number of 2-substituted 4,5-diphenylthiazoles were synthesized by the nucleophilic substitution of 2-methylsulfonyl-4,5-diphenylthiazole with various sodium alkoxides, amines, and carbanions of active methylene compounds. 2-Methylsulfonyl-4,5-diphenylthiazole was obtained by the potassium permanganate oxidation of 2-methylthio-4,5-diphenylthiazole in the presence of a phase-transfer catalyst. 2-Ethoxy-4,5-bis(4-methoxyphenyl) thiazole was prepared in a similar manner. 2-Ethynyl-4,5-diphenylthiazoles were synthesized by the palladium catalyst cross-coupling reaction of 2-iodo-4,5-diphenylthiazole with monosubstituted acetylenes. These compounds were tested for inhibitory activity against blood platelet aggregation in vitro. Among them, 2-alkoxy-, and 2-(4-methylpiperazin-1-yl)-4,5-diphenylthiazole were found to have potent inhibitory activity.     

Liver damage by the crown-of-thorns starfish (Acanthaster planci) lethal factor

Upon autopsy of mice injected with the crown-of-thorns starfish (Acanthaster planci) lethal factor, a change in color of the liver, swelling of the gall bladder and jaundice were observed. After administration of the lethal factor into mice, activities of glutamic oxaloacetic transaminase (GOT), glutamic pyruvic transaminase (GPT), lactate dehydrogenase (LDH), acid phosphatase (ACP) and alkaline phosphatase (ALP) were significantly elevated in serum. Hepatic ALP also increased markedly but hepatic GOT, GPT and ACP showed a tendency to decrease. Activity of hepatic LDH first decreased and then returned to control level. Histopathological studies showed that severe degenerative changes such as enlargement and necrosis of hepatocytes were caused in the mouse liver by the lethal factor. These results strongly suggested that the A. planci lethal factor is a potent hepatotoxin.     

Anterior spinal artery syndrome after minimally invasive direct coronary artery bypass grafting under general combined epidural anesthesia

A 65-year-old patient with ischemic heart disease and severe diabetes mellitus underwent minimally invasive direct coronary artery bypass grafting under general combined with epidural anesthesia. Paraplegia developed after surgery and the diagnosis of anterior spinal artery syndrome was made based on the patient s neurological condition and magnetic resonance imaging findings. Paraplegia following epidural anesthesia is a rare but recognized complication and this complication should be taken into account, especially in patients at risk, when considering epidural analgesia techniques in the minimally invasive cardiac surgery.     

Association of the GNAS1 gene variant with hypertension is dependent on alcohol consumption.

The beta-adrenoceptor (beta-AR)-stimulatory guanine nucleotide-binding (Gs) protein system has been shown to play important roles in the cardiovascular system. The gene encoding the alpha-subunit of Gs proteins (GNAS1) is a candidate genetic determinant for hypertension. Because alcohol consumption is known to affect blood pressure partly through the beta-AR-Gs protein system, we examined the possible interaction between GNAS1 T393C polymorphism and drinking status in the association with hypertension in the present study. As a result, a non-significant but reasonable trend supporting the presence of an interaction was shown (p = 0.076). In line with this trend, the T393C polymorphism significantly interacted with drinking status in the association with systolic blood pressure (p = 0.028). Moreover, supporting the presence of an interaction, T allele carriers consistently had a higher probability of hypertension, higher systolic blood pressure, and higher diastolic blood pressure than CC homozygotes in non-drinkers and light drinkers. In contrast, CC homozygotes consistently had a higher probability of hypertension, higher systolic blood pressure, and higher diastolic blood pressure than T allele carriers in moderate to heavy drinkers. The present study also showed a significant interaction between the T393C polymorphism and drinking status in the association with pulse pressure (p = 0.026), reflected by a significant association between the T393C polymorphism and pulse pressure in moderate to heavy drinkers (p = 0.026). These findings may be helpful in conducting further molecular and biological studies on the relationship among the effects of alcohol, the beta-AR-Gs protein system, and hypertension.     

Surgical treatment of aortic coarctation associated with multi-vessel brachiocephalic involvement in takayasu's arteritis.

In Takayasu's arteritis (TA), both atypical coarctation (CO) and brachiocephalic involvement are common features that occasionally require operative correction. A combination of these abnormalities could duplicate underlying illness in patients, posing an increased risk of operative morbidity. We present, herein, two TA patients in which hypertensive heart disease secondary to CO was surgically corrected. Both patients had multi-vessel brachiocephalic disease. One patient who showed occlusion of all brachiocephalic arteries underwent aorto-aortic bypass, while another with two-vessel lesion underwent axillo-bifemoral bypass grafting. Subclavian reconstruction was supplemental to each procedure, resulting in relief of neurologic stigmata. Strategies to avoid intraoperative cerebral ischemia played an important role in the surgical repair of such TA-related extensive vascular lesions.